An unusual case of acquired generalized lipodystrophy (panniculitis variety).

Acquired generalized lipodystrophy (AGL) is a rare disease characterized by variable loss of adipose tissue and concurrent metabolic derangements, typically with childhood or adolescent onset. AGL has three subclassifications: panniculitis (type 1), autoimmune disease (type 2), and idiopathic (type 3). This report highlights a rare case of AGL type 1 in a previously healthy 3-year-old female who presented with diffuse erythematous subcutaneous nodules, progressive lipoatrophy, and histopathological findings of a lobular panniculitis.

Immunohistochemical Staining of Pediatric Solitary Angiokeratomas.

The precise etiology and subtype of vessels constituting angiokeratomas is poorly understood. We sought to characterize the vessels by studying prospero-related homeobox gene-1 and D2-40 expression in 22 pediatric solitary angiokeratomas. Routine histologic examination demonstrated a mix of lymph-containing vessels and erythrocyte-filled small vessels. Our results suggest that angiokeratomas may in part be comprised of vessels with lymphatic differentiation.

Effectiveness of Exercise on Functional Mobility in Adults with Cerebral Palsy: A Systematic Review.

Purpose: We identified evidence evaluating the effect of exercise on functional mobility in adults (aged 18 y or older) with cerebral palsy (CP). Method: An exhaustive search was conducted using the electronic databases PubMed, MEDLINE, CINAHL, PsycINFO, SPORTDiscus, and Cochrane Database of Systematic Reviews from the earliest available evidence (1975) to the present (January 2016) for studies whose participants were ambulatory adults with CP receiving conservative treatment to address functional mobility limitations. Two independent reviewers agreed on the eligibility, inclusion, and level of evidence of each study. The Maastricht-Amsterdam List (MAL) was used to assess evidence quality. Results: Five of the six studies included were randomized controlled trials, and one was a pre-post case series. Interventions included whole-body vibration, treadmill training without body-weight support, rhythmic auditory stimulation, dynamic balance and gait activities, progressive resistance training, and interactive serious gaming for balance. All studies were considered high quality, as indicated by their MAL scores. Four studies showed no statistical difference and trivial effect sizes between the intervention and the control group. Rhythmic auditory stimulation and interactive serious gaming were found to be statistically significant in benefiting adults with CP. Conclusions: Evidence of the effect of exercise on functional mobility for ambulatory adults with CP is lacking. A need exists for quality research to determine the best interventions for adults with CP to maximize functional mobility.

Objectif : recenser les données probantes sur l'efficacité de l'exercice sur la mobilité fonctionnelle des adultes (18 ans et plus) atteints de paralysie cérébrale (PC). Méthode : une recherche exhaustive a été menée dans les bases de données électroniques PubMed, MEDLINE, CINAHL, PsycInfo, SPORTDiscus et Cochrane Database of Systematic Reviews à partir des données les plus anciennes (1975) jusqu'à aujourd'hui (janvier 2016) sur des études menées auprès d'adultes ambulatoires atteints de PC recevant un traitement conservateur pour leur mobilité fonctionnelle réduite. Deux examinateurs indépendants ont convenu de l'admissibilité, de l'inclusion et du niveau de données probantes de chaque étude. La liste de critères Maastricht-Amsterdam (MA) a été utilisée pour évaluer la qualité des preuves. Résultats : cinq des six études incluses étaient des essais randomisés et l'autre était une série de cas avant/après. Les interventions incluaient des vibrations transmises à l'ensemble du corps, un entraînement sur tapis roulant sans support du poids corporel, une stimulation auditive rythmique, des activités d'équilibre dynamique et de démarche, des exercices progressifs de musculation et des jeux interactifs d'équilibre. Toutes les études étaient de grande qualité, comme l'indique leur score de la liste de critères MA. Quatre études n'ont montré aucune différence statistique et ont montré une ampleur de l'effet négligeable entre l'intervention et le groupe témoin. La stimulation auditive rythmique et les jeux interactifs ont montré des bienfaits significatifs d'un point de vue statistique chez les adultes atteints de PC. Conclusions : il n'y a pas suffisamment de données probantes sur l'effet de l'exercice sur la mobilité fonctionnelle des adultes ambulatoires atteints de PC. Des recherches de qualité sont nécessaires pour déterminer les interventions les plus efficaces pour améliorer la mobilité fonctionnelle des adultes atteints de PC.

Crohn's disease presenting as a neutrophilic dermatosis in a 5-month-old boy.

A 5-month-old boy with a previous history of failure to thrive and poor feeding was admitted to the hospital with failure to thrive, oral ulcers, and a generalized vesiculopustular rash that demonstrated a subcorneal pustule and neutrophilic infiltrate on histology. Esophagogastroduodenoscopy and flexible sigmoidoscopy biopsies demonstrated chronic active colitis with granulomas, consistent with the diagnosis of Crohn's disease. Our case represents, to our knowledge, the youngest person reported with this condition in association with Crohn's disease.